Cystic fibrosis--1. Clinical aspects.

Cystic fibrosis is a fatal inherited disease involving many different organs. At 
one time most affected children died within a few years of 
birth but modern management has led to a marked improvement in 
prognosis and, of course, to an increase in the number of 
affected older children and young adults. Cystic fibrosis is, therefore, no 
longer a disease encountered only in paediatric units, but with increasing 
survival into teens and twenties affected patients are now appearing in 
adult units. The aetiology and pathology, the clinical picture, diagnosis, treatment, 
and prognosis are discussed. 